Primary mediastinal seminoma

Primary mediastinal seminoma

Primary mediastinal seminoma A case report and review of the literature Istvan Besznydk, M.D., Miklos Sebesteny, M.D., and Ferenc Kuchar, M.D., Budape...

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Primary mediastinal seminoma A case report and review of the literature Istvan Besznydk, M.D., Miklos Sebesteny, M.D., and Ferenc Kuchar, M.D., Budapest, Hungary

Mediastinal tumors of germinal cell origin are rare. The first tumor of this category, a mediastinal seminoma, was recognized by Friedman' in 1951. He compared it to a testicular seminoma or ovarian dysgerminoma. To date, 74 primary seminoma-like tumors of the anterior mediastinum have been reported (Table I). Recently we had the opportunity to operate upon a patient with a mediastinal seminoma. The present paper is a summary of our case which prompted a review of the pertinent literature. Case report P. H., a 44-year-old man, lost 6 kilograms in 3 months but had no other complaints. Physical examination was essentially negative. The external genitalia were normal for the patient's age; two normal testes were palpated in the scrotum. There was no evidence of gynecomastia or other endocrine abnormalities. Laboratory data, the electrocardiogram, respiratory tests, bronchoscopy, and pulmonary scintigraphy disclosed no abnormalities. Thoracic fluoroscopy revealed an apple-sized opacity on the left side at the level of the ascending aorta and the aortic arch. This structure appeared neither calcified nor cystic (Fig. 1). The mass indented the superior vena cava over a length of 3 to 4 cm. (Fig. 2). The thoracic and lumbar vertebrae, ribs, clavicles, and pelvic bones were within normal limits. Since the radiologic findings suggested a solitary mediastinal tumor, a left thoracotomy was done through the fourth intercostal space. At operation a firm, baseball-sized mass

From the Fourth (Cardiovascular) Surgical Clinic, Semmelweis Medical University, Budapest, Hungary.

Received for publication Feb. 21, 1973.

930

was found in the anterior superior mediastinum; this mass was encapsulated, smooth-surfaced, and was adherent to the apical portion of the lung. The mediastinal lymph nodes appeared normal. After careful dissection, a 150 gram mass was removed intact. The cut surface of the encapsulated neoplasm was firm, homogenous, and gray to yellow. Histologically, the tumor consisted of cell nests composed of sharply circumscribed round cells, 15 to 20 p. in diameter. These cells had a thin rim of pale cytoplasm and a large round or oval nucleus with well-defined, slightly eosinophilic nucleoli. Mitotic forms were rare. Syncytioblasts and cytotrophoblasts were not present. Teratomato us elements could not be found. Collections of lymphocytes were observed in the stroma, but no granulomatous reaction was noted. The microscopic diagnosis of a seminoma was made (Fig. 3). In view of this diagnosis, a postoperative radiologic work-up for skeletal metastases was done and no abnormalities could be recognized. After an uneventful recovery, the patient was discharged. He refused radiation therapy to the mediastinum but has been well and symptom free 2 years after removal of the tumor.

Comment Primary mediastinal seminomas occur most frequently in young men, the average age being 27 years." The symptoms are the same as with other mediastinal tumors, namely retrosternal pressure, hoarseness, brachial pain, loss of weight, matutinal swelling of the face, and, sometimes, superior vena cava syndrome. The tumor may compress and occasionally infiltrate the adjacent organs. Seminomas of the mediastinum are sometimes strikingly large" and may metastasize in the adjacent lymph nodes, to the

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Primary mediastinal seminoma

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Table I Case Nos.

Authors

Follow-up

1 2 3 4 5 6 7 8 9 10

Pugsley and Carleton?" Woolner et al. 3 2 Woolner et al.3~ Woolner et al.3~ Effler and McCormack" Effler and McCormack" Taniguchi et al."? Taniguchi et al. 3 1l O'Gara et al.~l O'Gara et al.~l

1953 1955 1955 1955 1956 1956 1957 1957 1958 1958

16, 24, 23, 31, 30, 32, 19, 18, ? 27,

M M M M M M M M

11 12 13 14 15 16 17 18 19 20

Tnada and Nakano!" Robinson>' Audier et al.' Steinmetz and Hays-? Kuznetsov et al. l G Kuznetsov et al." r;

Lattes! •

1959 1960 1960 1961 1961 1961 1962 1962 1962 1962

23, 35, 30, 35, 30, 26, 18, 20, 23, 34,

21 22 23 24 25 26 27 28 29 30

Kountz et aP' Kountz et al.' Kountz et al. l' Kountz et' alY' Inada et al.? Inada et al.v Oberman and Libcke-" Oberman and Libcke?" Pachter and Lattes'" Molina et al.J 8

1963 1963 1963 1963 1963 1963 1964 1964 1964 1965

31 32 33 34 35 36 37 38 39 40 41

Molina et al. l 8 Nazari and Gagnont" Shields et aL27 Kleitsch et aP' Edland et al.! Bagshaw et al." Bagshaw et aL2 Bagshaw et aI.2 Bagshaw et al." Bagshaw et al." Bagshaw et al.?

42 43 44 45-53 54-55 56-66 67 68 69-74

S R S S+R R R S S S+R S

Died 2 mo. Died 6 yr. Died 3 mo. Alive 7 yr. Alive 9 yr. Alive 6 yr. Died during operation Died during operation Alive 7 yr. Alive 7 yr.

M M M M M M M M M M

S+R R S+R R R

Alive Alive Alive Died Alive

8 yr. 2 yr. 3 yr. 1 yr., 6 mo. 10 mo.

S S S+R S+R

Alive Alive Alive Alive

7 4 2 2

33, 37, 27, 35, 23, 23, 42, 23, 46, 31,

M M M M M M M M M M

S+R S S+R S+R S S+R S+R S+R S+R S+R

Alive 15 mo. Alive 6 yr. Alive 10 mo. Asymptomatic, with metastases Alive 8 yr. Died 130 days Alive 5 yr. Died 14 mo. Died 6 mo. Alive 2 yr.

1965 1966 1966 1967 1968 1969 1969 1969 1969 1969 1969

62, 35, 26, 54, 20, 33, 27, 52, 21, 56, 35,

M M M M M M M M M M M

R S+R R S+R R R R R R S+R

1 mo. (autopsy) Alive 7 yr., 9 mo. Died 3 yr., 6 mo. Died 18 mo. Alive 1 yr. Alive 7 yr. Alive 6 yr., 4 mo. Alive 1 yr., 3 mo. Died 7 mo. Died 5 mo. Alive 6 yr., 3 mo.

Spitzer and Schmitt-" Inada et aI. Jl Hochholzer et aL8 EI-Domeiri et aI.G

1969 1969 1969 1968

34, M 28, M 28, M 17-42; 8 M, 1 F

Friedman' Iversont" Bariety and Coury> Joseph et aJ.l" Utz and Buscemi-t

1951 1956 1958 1966 1971

Lattes' t Lattes!?

Lattes'"

Legend: S, Surgery. R, Radiotherapy.

M

9 M, 2 F

S S S 1 S, 6 R, 2S+R

yr., 6 mo. yr., 6 mo. yr., 6 mo. yr.

Alive Alive 1 yr. ? Survival 1-18 yr. No No No No No

details details details details details

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The Journa l of Tho rac ic a nd Cardiovas cular Surgery

Fig. 1. Posteroanterior chest roentgenogram showing left anterior mediastinal mass.

Fig. 2. Venogram showing partial obstruction and dislocation of superior vena cava.

Fig. 3. High-power view of the mediastinal seminoma. (Hematoxylin and eosin; original mag nification x240.)

lungs, spleen, tonsils, thyroid gland, suprarenal glands , bones, skin, or spinal cord. Although the histogenesis of these neoplasms is not clear, Friedman' postulated that the mediastinal teratomas originate in extragonadal germ cells within the thymus . According to Friedman, seminomas are at the earlie st and simplest stage of development among these tumors, the next step being embryonal carcinomas which may de-

velop into teratomas and /or chorionepitheliomas. According to Iverson,'? aberrant germ cells may be included in an anomalous thymus, and these cells may later give rise to the tumors. Schlumberger'": 2 6 suggested that extragonadal germinal tumors develop from germ cell layers dislocated during embryonic life. The presence of residual thymic tissue intimately associated within the tumor certainly supports this hypothesis ." Others

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have proposed that primary testicular tumors may regress spontaneously while their mediastinal metastases continue to grow. However, the long follow-up of many of these patients and the absence of palpable testicular masses or retroperitoneal or paraaortic metastases favor the primary nature of the mediastinal tumors. The tumor is histologically identical to the testicular seminomas.': IH, 27 With regard to the histogenesis, we agree with Lattes" that most of what has been written is purely speculative; however, sufficient data are available to establish this neoplasm as a separate pathologic entity. Although permanent cure has been achieved by surgery alone, the tumor frequently is not resectable. In less advanced cases, total surgical resection should be followed by radiation therapy." As with testicular seminomas, metastatic spread does not rule out the possibility of cure. The rate of I year survival after combined surgical and radiation therapy is 67 per cent." Summary A primary seminoma was removed from the mediastinum of a 44-year-old man. The patient has been symptom free for 2 years after the operation. The pathogenesis, symptomatology, and therapy of this rare tumor are briefly discussed. The present case is the seventy-fourth in the world literature. REFERENCES Audier, M., Dor, J., Picard, D., Payan, H., Clement, L. G., and Galinier, L.: Thymome "pseudo-serninorne," Presse Med. 68: 574, 1960. 2 Bagshaw, M. A., McLaughlin, W. T., and Earle, J. D.: Definitive Radiotherapy of Primary Mediastinal Seminoma, Am. J. Roentgenol. Radium Ther. Nucl. Med. 105: 86, 1969. 3 Bariety, M., and Coury, C.: Le rnediastin et sa pathologie, Paris, 1958, Masson & Cie, Editeurs, p. 228. 4 Edland, R. W., Levine, S., Serfas, L. S., and Flair, R. C.: Seminoma-like Tumor in the Hyperplastic Thymus Gland: A Case Report and Literature Review, Am. 1. Roentgenol. Radium Ther. Nucl. Med. 103: 25, 1968.

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5 Effler, D. B., and McCormack, L. J.: Thymic Neoplasms, 1. THORAC. SURG. 31: 60, 1956. 6 El-Domeiri, A. A., Hutter, R. V. P., Pool, J. L., and Foote, F. W., Jr.: Seminoma of the Anterior Mediastinum, Ann. Thorac. Surg. 6: 513, 1968. 7 Friedman, N. B.: The Comparative Morphogenesis of Extragenital and Gonadal Teratoid Tumors, Cancer 4: 265, 1951. 8 Hochholzer, L., Theros, E. G., and Rosen, S. H.: Some Unusual Lesions of the Mediastinum: Roentgenologic and Pathologic Features, Scm. Roentgeno!. 4: 74, 1969. 9 Inada, K, Kawasaki, A., and Harnazaki, M.: Germinoma of the Mediastinum, Am. Rev. Resp. Dis. 87: 560, 1963. 10 Inada, K, and Nakano, A.: Germinoma of the Mediastinum: A Case With Special Reference to the Histogenesis of Mediastinal Teratoma, Chest 36: 438, 1959. 11 Inada, K., Yamada, R., Fu, S., and Harnazaki, M.: Primary Mediastinal Seminoma: A Case Report With Review of Literature, Jap. J. Thorac. Surg. 22: 442, 1969. 12 Iverson, L.: Thymoma: A Review and Reclassification, Am. J. Patho!. 32: 695, 1956. 13 Joseph, W. L., Murray, J. F., and Mulder, D. G.: Mediastinal Tumors: Problems in Diagnosis and Treatment, Chest 50: 150, 1966. 14 Kleitsch, W. P., Taricco, A., and Haslam, G. J.: Primary Seminoma (Germinoma) of Mediastinum, Ann. Thorac. Surg. 4: 249, 1967. 15 Kountz, S. L., Connolly, 1. E., and Cohn, R.: Seminoma-Like (or Seminomatous) Tumors of the Anterior Mediastinum: Report of Four New Cases and a Review of the Literature, J. THORAC. CARDIOVASC. SURG. 45: 289, 1963. 16 Kuznetsov, I. D., Lavnikova, G. A., and Koroleva, O. F.: Two Cases of Seminoma of the Mediastinum, Vopr. Onkol. 7: 55, 1961. 17 Lattes, R.: Thymoma and Other Tumors of the Thymus, Cancer 15: 1224, 1962. 18 Molina, C,; Mercier, R., Delage, J., deLaguillaumie, B., and Cheminat, J. C.: Les serninornes du mediastin, Pre sse Med. 41: 1416, 1965. 19 Nazari, A., and Gagnon, E. D.: SeminomaLike Tumor of the Mediastinum: A Case Report With 7 Year Follow-Up, 1. THORAC. CARDIOVASC. SURG. 51: 751, 1966. 20 Oberman, H. A., and Libcke, 1. H.: Malignant Germinal Neoplasms of the Mediastinum, Cancer 17: 498, 1964. 21 O'Gara, R. W., Horn, R. C., Jr., and Enterline, H. T.: Tumors of Anterior Mediastinum, Cancer 11: 562, 1958. 22 Pachter, M. R., and Lattes, R.: "Germinal" Tumors of the Mediastinum: A Clinicopathologic Study of Adult Teratomas, Teratocarcinomas, Choriocarcinomas and Seminomas, Chest 45: 301, 1964.

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23 Pugsley, W. S., and Carleton, R. L.: Germinal Nature of Teratoid Tumors of the Thymus, Arch. Pathol. 56: 341, 1953. 24 Robinson, B. W.: Germinal Neoplasia of Extragenital Origin, J. Natl. Med. Assoc. 52: 162, 1960. 25 Schlumberger, H. G.: Teratoma of Anterior Mediastinum in Group of Military Age: Study of 16 Cases and Review of Theories of Genesis, Arch. Pathol. 41: 398, 1946. 26 Schlumberger, H. G.: Tumors of Mediastinum, in Atlas of Tumor Pathology, Section Y, Fascile 18, Washington, D. C., 1951, Armed Forces Institute of Pathology. 27 Shields, T. W., Fox, R. T., and Lees, W. M.: Thymic Tumors: Classification and Treatment, Arch. Surg. 92: 617, 1966. 28 Spitzer, G., and Schmitt, W.: Uber ein extra-

29

30

31 32

gonadales Seminom im Mediastinum, Zentralbl. Chir. 94: 646, 1969. Steinmetz, W. H., and Hays, R. A.: Primary Seminoma of the Mediastinum. Report of a Case With an Unusual Site of Metastasis and Review of the Literature, Am. J. Roentgenol. Radium Ther. Nucl. Med. 86: 669, 1961. Taniguchi, H., Pai, T. J., and Amakata, Y.: Two Cases of Seminomatous Tumors Originating from the Anterior Mediastinum, Gann 48: 639, 1957. Utz, D. C., and Buscemi, M. F.: Extragonadal Testicular Tumors, J. Urol. 105: 271, 1971. Woolner, L. B., Jamplis, R. W., and Kirklin, J. W.: Seminoma (Germinoma) Apparently Primary in the Anterior Mediastinum, N. Engl. J. Med. 252: 653, 1955.